| 555 | For the treatment of Heterozygous Familial Hypercholesterolemia (HeFH) in patients 18 years of age or older who meet the following criteria:
1. Definite or probable diagnosis of HeFH using the Simon Broome or Dutch Lipid Network criteria or genetic testing;
AND
2. Unable to reach Low Density Lipoprotein Cholesterol (LDL-C) target (i.e., LDL-C less than 2.0 mmol/L for secondary prevention) or at least a 50% reduction in LDL-C from untreated baseline for primary prevention despite:
A) Confirmed adherence to ezetimibe for at least a total of 3 months in combination with high dose statin (e.g., atorvastatin 80mg or rosuvastatin 40mg);
OR
B) Confirmed adherence to ezetimibe for at least a total of 3 months and inability to tolerate high dose statin defined as:
(i) Inability to tolerate at least 2 statins with at least one started at the lowest starting dose;
(ii) For each statin (two statins in total), dose reduction is attempted for intolerable symptom (myopathy) or biomarker abnormality (creatine kinase (CK) greater than 5 times the upper limit of normal) resolution rather than discontinuation of statin altogether;
(iii) For each statin (two statins in total), intolerable symptoms (myopathy) or abnormal biomarker (creatine kinase (CK) greater than 5 times the upper limit of normal) changes are reversible upon statin discontinuation but reproducible by re-challenge of statins where clinically appropriate; and
(iv) One of the following:
(I.) Other known determinants of intolerable symptoms or abnormal biomarkers have been ruled out;
(II.) Patient developed confirmed and documented rhabdomyolysis;
(III.) Patient is statin contraindicated i.e. active liver disease, unexplained persistent elevations of serum transaminases exceeding 3 times the upper limit of normal.
Treatment with alirocumab should be discontinued if the patient does not meet all of the following:
1. Patient is adherent to therapy.
2. Patient has achieved a reduction in LDL-C of at least 40% from baseline (4-8 weeks after initiation of alirocumab).
3. Patient continues to have a significant reduction in LDL-C (with continuation of alirocumab) of at least 40% from baseline since initiation of PCSK9 inhibitor. LDL-C should be checked periodically with continued treatment with PCSK9 inhibitors (e.g., every 6 months).
Patients prescribed alirocumab 75mg every two weeks must use the 75mg/mL dosage strength and are limited to 26 pre-filled pens (PFP) per year.
Patients prescribed alirocumab 150mg every two weeks must use the 150mg/mL dosage strength and are limited to 26 PFP per year.
Patients prescribed alirocumab 300mg every four weeks may use either the 150mg/mL dosage strength (limited to 26 PFP per year) or the 300mg/2mL dosage strength (limited to 13 PFP per year). |
